[Alagille syndrome, a rare differential diagnosis of intrahepatic cholestasis].
1987
In Alagille's syndrome cholestasis is caused by a congenital hypoplasia of the intrahepatic bile ducts. If cardiac and arterial malformations are present in conjunction with the typical-but not pathognomonic-facies, this rare syndrome must be considered. ERCP shows a highly rarified bile duct system. Liver histology with a paucity of interlobular bile ducts establishes the diagnosis. Inheritance appears to be autosomal dominant. The relatively good prognosis of the disease calls for caution concerning invasive procedures.
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